Abstract
Frontotemporal lobar degeneration (FTLD) is a neuropathologically and genetically heterogeneous group of neurodegenerative syndromes leading to dementia and primarily affecting frontal and temporal lobes. FTLD patients can develop non-cognitive symptoms in keeping with amyotrophic lateral sclerosis or parkinsonian syndromes. This wide clinical spectrum is explained to some extent with similar pathological and genetic backgrounds. FTLD shows a strong familial component, with up to 50 % of cases having a positive family history. So far, mutations in seven genes are known to cause autosomal dominant FTLD. Mutations in the genes microtubule-associated protein tau and progranulin, and the most recently identified hexanucleotide repeat expansion within C9ORF72 cover most of the genetically identified cases. Recent advances in molecular genetics and neuropathological characterization of FTLD have provided valuable insights into the pathogenic mechanisms of FTLD and opened possibilities to identify new biomarkers and therapeutic targets for the diagnostics and treatment of this disease.
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